Session 5: Movement Disorders. Vignette 55 yo accountant: –Frozen right shoulder/stiffness...

Session 5:Movement Disorders

Vignette

• 55 yo accountant:– Frozen right shoulder/stiffness– Slowly progressive– Impaired fine motor function– Right hand tremor– Reduced right arm swing– Slow overall– ? Sexual function– ? Bladder function– ? Cognitive function– ? Falls– ? Hallucinations– ? Medications or drug exposure– ? Family history

Examination

• No orthostatic hypotension• Hypomimia• Hypophonia• Normal eye movements• RUE and neck rigidity• Right hand and jaw tremor• Reduced right arm swing• Slight impairment in postural stability

Some Causes of ParkinsonismSome Causes of Parkinsonism

MSA

PDPSP

DLBD

VPPPFG

CBD

FTD

MedsNPH

AD

Parkinsonism DDX• Parkinson’s disease (PD)• Multiple System Atrophy (MSA)• Progressive Supranuclear Palsy (PSP)• Medication-induced parkinsonism

– Antipsychotics– Metoclopramide– Others

• Parkinsonism with Alzheimer’s Disease (AD)• Diffuse Lewy Body Disease (DLBD)• Vascular parkinsonism (VP)• Corticobasal Degeneration (CBD)• Normal Pressure Hydrocephalus (NPH)• Gait disorder of the elderly• Toxins: CO, CN, Manganese, MPTP, rotenone?, CS2,

Red Flags if Seen Early

• Falls

• Cognitive changes

• Prominent sexual or bladder dysfunction

• Lack of response to adequate Rx trial

• Symmetric onset

• Early bulbar dysfunction

Red Flags at Any TimeRed Flags at Any Time

Cortical Sensory SignsCortical Sensory Signs

Alien Limb/unilateral apraxiaAlien Limb/unilateral apraxia

No response to L-dopaNo response to L-dopa

Impaired vertical eye movements Impaired vertical eye movements

Dementia

Parkinsonism

Dementia

Dementia

DementiaParkinsonism

Parkinsonism

Initial Symptoms

AD

DLBD

PDD

Years Later

Diffuse Lewy Body Disease Video

Click here to view movie

Progressive Supranuclear Palsy

Dudley Moore

• Supranuclear palsy• Falls early in course• Dysarthria• Cognitive impairment• Emotional

incontinence• Abducted arms when

ambulating: “gun-slinger’s gait”

PSP: Abnormal PSP: Abnormal Voluntary Eye MovementsVoluntary Eye Movements

Baylor U

ParkinsonismParkinsonism

TTremorremor = rhythmical; typically a rest tremor = rhythmical; typically a rest tremor

RRigidityigidity = resistance to movement = resistance to movement

AAkinesiakinesia = slowness/absence of movement = slowness/absence of movement

PPostural changesostural changes = stooped posture = stooped posture

Resting TremorClick here to view movie

RigidityClick here to view movie

Akinesia/BradykinesiaClick here to view movie

Postural Changes

Stooped Posture

www.artandmedicine.com

Impaired Postural Reflexes

Click here to view movie

PD:Preferential loss of

putamenal dopamine projections

Dauer and PrzedborskiNeuron 2003

Lewy bodies containsynuclein, ubiquitin and other proteins

Normal PD

Increased Tone• Rigidity

– Increased tone– Usually bidirectional

• Paratonia (Gegenhalten)– Inability to relax one’s limb manifesting as

resistance to movement

• Spasticity– Velocity dependent increase in tone– Usually unidirectional– Other upper motor neuron signs are often present:

hyper-reflexia, Babinski sign and pyramidal distribution of weakness

Definitions• Chorea: derived from the Greek word “to dance” to

describe the arrhythmic, involuntary flitting movements• Athethosis: involuntary writhing movements• Hemiballism: large amplitude involuntary movement

restricted to one side of the body; usually involves proximal upper limb.

• Myoclonus: sudden brief jerk or shock-like movements• Dystonia: abnormal sustained posture resulting from

simultaneous co-contraction of agonist and antagonist muscles.

• Tremor: rhythmic oscillation of a body part due to alternating or synchronous contractions of opposing muscles

• Tics: sudden, brief, purposeless, stereotyped simple or complex movements or vocalizations

• Akathisia: inner restlessness; often associated with external signs of restless behavior

ET TreatmentsET Treatments

MedicationsMedicationsPropranolol (InderalPropranolol (Inderal®®))

Mysoline (PrimidoneMysoline (Primidone®®))

Clonazepam (KlonopinClonazepam (Klonopin®®))

SurgeriesSurgeriesThalamotomyThalamotomy

Thalamic Deep Brain Stimulation Thalamic Deep Brain Stimulation (DBS)(DBS)

Tremor

• Cerebellar disorders– Tremor with action with accompanying dysmetria.

Note cerebellar features on exam: asynergia, ataxia, dysarthria, dysdiadochokinesia, dysmetria, gait disturbance, hypotonia, nystagmus, rebound, etc.

• Essential tremor– Postural and action tremor without parkinsonism;

typically life-long and tremor responds to alcohol; may have family history

• Parkinsonism– Typically has a rest tremor, but may also be present

with posture and action or solely the latter

Acute Neuroleptic-induced A/Es

• Acute dystonic reaction– Cervical dystonia (torticollis)– Opisthotonus– Oculogyric crisis– Other

• Neuroleptic malignant syndrome

• Akathisia (restlessness)

Acute Dystonic Reaction

• Usually occurs within 48 hours; but sometimes may be up to 5 days after Rx

• More common with parenteral than oral medications

• More common in males than females; particularly young males

• Features: neck, jaw, tongue, oculogyric, opisthotonus, etc.

• Rx: anticholinergics, antihistaminics

Acute Dystonic Reaction

http://www.haveinc.com/eps/

Opisthotonus

www.pharmacology2000.com/ Central/sedhyp/opis1.jpg

Akathisia: Restlessness

http://www.haveinc.com/eps/

Chronic Neuroleptic Exposure

• Tardive dystonia– Axial more common– Appendicular (limb)

• Tardive dyskinesia

• Medication induced parkinsonism

• Tardive tics/akathisia/tremor, etc.

73 yo woman with action tremor

• DDx– Essential tremor (ET)

• Life-long, progressive tremor• Alcohol responsiveness• Absence of bradykinesia, rigidity, etc.

– Parkinson syndrome with action tremor– Medication induced tremor– Could be both ET and PD

56 yo man with progressive chorea, dementia and positive family history

Huntington’s DiseaseHuntington’s Disease

Features:Features:ChoreaChorea

Cognitive declineCognitive decline

Cause:Cause:

Autosomal dominant Autosomal dominant

Amplification of CAG (cytosine adenosine Amplification of CAG (cytosine adenosine guanine) repeats in the IT-15 gene on guanine) repeats in the IT-15 gene on chromosome 4chromosome 4

SOME TRINUCLEOTIDE REPEAT DISORDERS

HD occurs with > 36-38 CAG repeats in the IT15 gene

ANTICIPATION: EARLIER ONSET WITH MORE CAG REPEATS

AUTOSOMALDOMINANT

TRANSMISSION

32 yo man with recurrent grunting, OCD and unusual involuntary limb

movements

Tourette’s Syndrome (TS)Tourette’s Syndrome (TS)Tics:

Involuntary stereotypic movements or vocalizationsTS Features:

Presence of both vocal and motor tics Onset before age 18/21 yearsTics may be temporarily suppressibleWith suppression of tics there is often inner tension which is relieved upon performing ticsCoprolalia (foul language): only ~17% of casesCo-morbidities: OCD, ADHD, learning disability

54 year-old man who reacts to metaclopramide

• Age

• Gender

• Other factors